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PANK2 Antikörper (AA 401-500) (AbBy Fluor® 555)

PANK2 Reaktivität: Maus WB, IF (cc), IF (p) Wirt: Kaninchen Polyclonal AbBy Fluor® 555
Produktnummer ABIN1398503
  • Target Alle PANK2 Antikörper anzeigen
    PANK2 (Pantothenate Kinase 2 (PANK2))
    Bindungsspezifität
    • 14
    • 7
    • 7
    • 6
    • 4
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 401-500
    Reaktivität
    • 41
    • 20
    • 5
    • 4
    • 4
    • 4
    • 4
    • 3
    • 3
    • 3
    • 3
    • 3
    • 3
    • 1
    Maus
    Wirt
    • 49
    • 5
    Kaninchen
    Klonalität
    • 50
    • 5
    Polyklonal
    Konjugat
    • 22
    • 5
    • 4
    • 4
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    Dieser PANK2 Antikörper ist konjugiert mit AbBy Fluor® 555
    Applikation
    • 43
    • 25
    • 12
    • 12
    • 7
    • 7
    • 5
    • 3
    • 3
    • 1
    • 1
    • 1
    Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))
    Kreuzreaktivität
    Maus
    Homologie
    Human,Rat,Dog,Cow,Pig,Horse
    Aufreinigung
    Purified by Protein A.
    Immunogen
    KLH conjugated synthetic peptide derived from human PANK2
    Isotyp
    IgG
    Top Product
    Discover our top product PANK2 Primärantikörper
  • Applikationshinweise
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • Format
    Liquid
    Konzentration
    1 μg/μL
    Buffer
    Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.
    Konservierungsmittel
    ProClin
    Vorsichtsmaßnahmen
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    Lagerung
    -20 °C
    Informationen zur Lagerung
    Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
    Haltbarkeit
    12 months
  • Target
    PANK2 (Pantothenate Kinase 2 (PANK2))
    Andere Bezeichnung
    PANK2 (PANK2 Produkte)
    Synonyme
    ATPANK2 antikoerper, F10M6.180 antikoerper, F10M6_180 antikoerper, pantothenate kinase 2 antikoerper, PANK2 antikoerper, C20orf48 antikoerper, HARP antikoerper, HSS antikoerper, NBIA1 antikoerper, PKAN antikoerper, 4933409I19Rik antikoerper, AI642621 antikoerper, pantothenate kinase 2 antikoerper, pantothenate kinase 2, mitochondrial antikoerper, ribonuclease A family member 11 (inactive) antikoerper, PANK2 antikoerper, Tsp_01576 antikoerper, LOC100539295 antikoerper, Pank2 antikoerper, RNASE11 antikoerper
    Hintergrund

    Synonyms: Pantothenate kinase 2, mitochondrial, hPanK2, Pantothenic acid kinase 2, PANK2, C20orf48, PANK2_HUMAN

    Background: Defects in PANK2 are the cause of neurodegeneration with brain iron accumulation type 1 (NBIA1), also known as pantothenate kinase-associated neurodegeneration (PKAN) or Hallervorden-Spatz syndrome (HSS). It is an autosomal recessive neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. Clinical manifestations include progressive muscle spasticity, hyperreflexia, muscle rigidity, dystonia, dysarthria, and intellectual deterioration which progresses to severe dementia over several years. It is clinically classified into classic, atypical, and intermediate phenotypes. Classic forms present with onset in the first decade, rapid progression, loss of independent ambulation within 15 years. Atypical forms have onset in the second decade, slow progression, maintenance of independent ambulation up to 40 years later. Intermediate forms manifest onset in the first decade with slow progression or onset in the second decade with rapid progression. Patients with early onset tend to also develop pigmentary retinopathy, whereas those with later onset tend to also have speech disorders and psychiatric features. All patients have the 'eye of the tiger' sign on brain MRI.Defects in PANK2 are the cause of hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration (HARP). HARP is a rare syndrome with many clinical similarities to NBIA1.

    Gen-ID
    80025
    UniProt
    Q9BZ23
    Pathways
    Ribonucleoside Biosynthetic Process
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